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Objective:To analyze the clinical characteristics and prognostic factors of high-risk neuroblastoma (HR-NB) patients with skeletal metastasis.Methods:The clinical features of 336 newly treated HR-NB patients with skeletal metastases admitted to the Department of Medical Oncology of Beijing Children′s Hospital, Capital Medical University from January 2007 to December 2018 were analyzed retrospectively.Kaplan-Meier method was used for the survival analysis, and Log- Rank test was used for univariate prognosis analysis.The Cox model was used to analyze the multifactorial prognostic analysis. Results:A total of 336 HR-NB patients were recruited, involving 188 males and 148 females with the median age of onset of at 43 (4-148) months.Skeletal metastases affected the viscerocranium (89 cases, 26.5%), neurocranium (193 cases, 57.4%), vertebrae (298 cases, 88.7%), sternum and ribs (183 cases, 54.5%), pelvis (270 cases, 80.4%), upper limbs (182 cases, 54.2%) and lower limbs (240 cases, 71.4%). The 5-year event-free survival (EFS) rate and overall survival (OS) rate were (30.4±2.7)% and (41.3±2.9)%, respectively.Univariate analysis showed a significantly lower 5-year OS rate in skeletal metastatic HR-NB patients with poor prognostic classification, the morphology of neuroblastoma (stroma-poor) and ganglioneuroblastoma (intermixed), high index of mitosis-karyorrhexis index, lactate dehydrogenase≥587 U/L, serum ferritin≥92 μg/L, MYCN amplification and 1p loss of heterozygosity, and metastases in the viscerocranium, neurocranium, vertebrae, sternum and ribs, pelvis, upper limbs and lower limbs (all P<0.05). The 5-year OS rate of HR-NB patients with all 7 regions of skeletal metastases was only (14.2±5.9)%, which was significantly lower than that in patients with a single region metastasis or multi-region metastases[(66.0±10.2)% vs.(43.6±3.4)%, χ2=45.722, P<0.05]. Cox multifactorial analysis showed that MYCN amplification ( HR=4.165, 95% CI: 2.356-7.363) and the viscerocranium metastasis ( HR=2.560, 95% CI: 1.519-4.315) were the independent risk factors affecting the prognosis of HR-NB patients with skeletal metastases (all P<0.05). Conclusions:The prognosis is extremely poor in HR-NB patients with multiple skeletal metastases at the initial diagnosis.The amplification of MYCN and the viscerocranium metastasis are the poor prognostic factors for HR-NB patients with skeletal metastases.
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Objective:To evaluate the long-term health-related quality of life (QOL) in pediatric patients with acute leukemia after hematopoietic stem cell transplantation (HSCT) and to analyze potential influence factors.Methods:Patients with acute leukemia aging 8-18 years who received HSCT in the Hematology Oncology Center of Beijing Children′s Hospital from June 2009 to June 2012 with more than 80 months survival postoperatively were recruited.All of them were subjected to a short-term QOL survey in 2013.PedsQL? Transplantation Module 3.0 in Chinese mandarin version was completed.QOL data and influence factors were analyzed.Results:Forty-one patients completed the questionnaires, involving 32 males and 9 females with the mean age of(14.29±2.72) years.The mean scores of overall long-term QOL after HSCT were above 75 (total scores: 100), which was above the average.The age, disease status before transplantation, donor sources, post-transplant complications and the parental education level were the influential factors for the long-term QOL in pediatric patients with acute leukemia at post-HSCT, which could affect a certain dimension in QOL.Conclusions:The overall long-term QOL of pediatric patients with acute leukemia who survived for more than 80 months at post-HSCT is acceptable, which is significantly better than the short-term QOL after 4 months of HSCT.The age, disease status before transplantation, donor sources, post-transplant complications and the education level of parents could affect a certain dimension of QOL.
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Objective To retrospectively analyze the clinical efficacy,safety and influencing factors of radiotherapy in children with stage Ⅳ high-risk neuroblastoma (HR-NB).Methods A total of 120 children with HR-NB who were diagnosed and treated with local radiotherapy according to the BCH-HR-NB-2007 protocol in the Oncology Department of Beijing Children's Hospital from January 2014 to December 2017 were enrolled.Among them,56 children were male and 64 female with a median age of 43 months (9 -148 months).The treatment protocol consisted of 4 cycles of CAV chemotherapy,3 cycles of CVP chemotherapy,surgical resection after 4 cycles,autologous hematopoietic stem cell transplantation after 7 cycles,local radiotherapy at a dose of 15.0-30.6 Gy for 82 cases of primary tumors and 38 cases of primary and metastatic tumors,followed by 13 cis-retinoic acid as maintenance therapy.The entire treatment protocol endured for approximately 18 months.Results The median follow-up time was 21 months.The 3-year local control rate was 84.4％.Before radiotherapy,the 3-year event-free survival rate was 78.4％ in children without metastases,significantly higher compared with 30.4％ in the residual group (P=0.003).The 3-year event-free survival rate was 66.1％ in patients who underwent radiotherapy within 6 months after surgery,significantly higher than 50.6％ in their counterparts receiving radiotherapy at 6 months or more after surgery (P=0.018).Among the children with residual metastases before radiotherapy,the progression rate in children who did not receive radiotherapy was 66.6％,significantly higher compared with 20.0％ in those receiving radiotherapy (P=0.001).All patients had no radiation-related adverse reactions in the liver,kidney and heart,etc.The incidence rate of grade Ⅲ-Ⅳ myelosupression was 24.5％ at 1 week post-radiotherapy,and 8％ at 2 weeks after radiotherapy.Conclusions Radiotherapy yields definite clinical efficacy in the local control of children with stage Ⅳ HR-NB.Early radiotherapy after surgery and radiotherapy for the metastatic lesions can improve the clinical prognosis.No vital organ injuries are observed during the short-term follow-up.At 2 weeks after radiotherapy,the myelosupression is gradually restored.
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OBJECTIVE@#To compare the efficacy and safety of different antiviral and antifibrotic regimens in patients with chronic hepatitis B (CHB) and hepatic fibrosis and the incidence of hepatocellular carcinoma (HCC) associated with these therapies.@*METHODS@#A total of 840 patients with CHB and concurrent hepatic fibrosis, who received antiviral therapy in Nanfang Hospital between June, 2010 and June, 2018, were enrolled in this follow-up cohort study. The patients were assigned to 3 cohorts matched for gender, age (difference≤5 years), HBeAg status and liver stiffness measurement (LSM) for treatment with one of the 3 antiviral drugs, namely entecavir, tenofovir dipivoxil and adefovir dipivoxil; each cohort was divided into 2 groups, with one of the groups having a combined treatment with Fufang Biejiaruangan tablet. The cumulative negative conversion rate of HBV DNA, normalization rate of ALT, hepatic fibrosis regression and the incidence of HCC were compared among the 3 cohorts and across the 6 groups at 144 weeks.@*RESULTS@#A total of 749 patients were available to follow-up at 144 weeks. Compared with the baseline data, the cumulative negative conversion rate of HBV DNA increased gradually and the abnormal rate of ALT decreased significantly over time during the treatment in all the 6 groups (all < 0.001). Compared with the any of the antiviral drugs used alone, the combined treatments all resulted in significantly better antifibrotic effects (χ=11.345, χ=10.160, χ=6.358; all < 0.05). At 144 weeks, the incidence of HCC were 2.2%, 1.7%, 1.7% and 3.3% in enecavir group, enecavir with Biejiaruangan tablet group, adefovir group, and adefovir with Biejiaruangan tablet group, respectively, showing no significant difference between the two cohorts (4 groups; χ=6.813, =0.138). None of the patients in the 2 groups with tenofovir treatment had HCC by the end of the observation.@*CONCLUSIONS@#Antiviral therapy combined with antifibrotic therapy can effectively reverse hepatic fibrosis and reduce the incidence of HCC in patients with CHB; among the 3 antiviral drugs, tenofovir dipivoxil can be a better option for reducing the incidence of HCC in these patients.
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Humans , Antiviral Agents , Carcinoma, Hepatocellular , DNA, Viral , Follow-Up Studies , Hepatitis B e Antigens , Hepatitis B virus , Hepatitis B, Chronic , Liver Cirrhosis , Liver Neoplasms , Prospective StudiesABSTRACT
OBJECTIVE@#To analyze the incidence and risk factors of benign liver space-occupying mass in patients with chronic hepatitis B (CHB) and the ultrasound features that differentiate these masses from small hepatocellular carcinoma.@*METHODS@#We retrospectively analyzed the color Doppler and clinical data of 17 721 patients with CHB treated in the Hepatology Unit of Nanfang Hospital between January, 2016 and December, 2017. The data were compared with those of 21629 healthy control subjects undergoing routine physical examination in the Center of Heath Management of Nanfang Hospital during the same period.@*RESULTS@#Compared with the control subjects, the patients with CHB had significantly higher incidences of hepatic cysts (11.8% 8.7%, 0.05). Sonographically, the benign liver masses commonly showed homogeneous echo within the lesion with clear boundaries and regular shape. Hepatic hemangioma was distinctively hyperechoic in 83.32% (1579/1895) of the patients, while small hepatocellular carcinoma presented with weaker peripheral and internal blood flow signals with a lower flow velocity in the arteries and a higher flow velocity in the portal vein. Liver cirrhosis nodules mostly showed a mixture of strong and weak echoes (79.60%; 7637/9595) without blood flow signal within or around the nodule; an increased volume of the nodule accompanied by heterogeneous echoes within the nodule indicated an increased probability of malignant lesion. Hepatic cysts often displayed no echo within the lesion, but the echo could be enhanced posteriorly.@*CONCLUSIONS@#The patients with CHB are at a significantly higher risk of developing hepatic cysts, hepatic hemangiomas and hepatic cirrhosis nodules than the control population, and an older age and the male gender are associated with a higher incidence of hepatic cysts or cirrhosis. The differences in the sonographic and hemodynamic features can help to differentiate hepatic benign mass from malignant lesions, and kinetic changes in sonography can be used to monitor potential malignant transformation of the cirrhotic lesions.
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Objective@#To analyze the clinical characteristics of newly treated high-risk group neuroblastoma (NB) patients with bone marrow metastasis and to explore the prognostic factors.@*Methods@#The clinical features (sex, age, stage, risk group, pathological type, metastatic site, etc.) of 203 newly treated high-risk NB patients with bone marrow metastasis admitted to Hematology Oncology Center, Beijing Children′s Hospital from January 2007 to December 2016 were analyzed retrospectively. There were 118 males (58.1%) and 85 females (41.9%). Kaplan-Meier method was used for survival analysis and Cox regression was used to analyze the prognostic factors.@*Results@#The age at onset of the 203 patients was 41 months (9-147 months). The metastatic sites at diagnosis were as follows: bone in 195 cases (96.1%), distant lymph nodes in 104 cases (51.2%), skull and endomeninx in 61 cases (30.0%), orbit in 30 cases (14.8%), pleura in 16 cases (7.9%), liver in 13 cases(6.4%), canalis spinalis in 13 cases (6.4%), other sites in 11 cases (5.4%) and skin and soft tissue in 10 cases (4.9%). In all, 194 cases were enrolled for prognostic analysis. The follow-up time was 36 months (1 day-138 months) , and the 5-years event free survival (EFS) and overall survival (OS) were 36.1% and 39.7%, respectively. A total of 118 patients (60.8%) had events (first relapse or death) with the time to event occurrence was 15 months (1 day-72 months), whereas 112 patients (57.7%) died with the event occurrence to death time was 3 months (1 day-21 months). There was no significant difference in 5-years OS between radiotherapy group and non-radiotherapy group (42.3% vs. 38.3%, χ2=3.671, P=0.055). The 5-years OS in transplantation group was significantly better than the non-transplantation group (44.3% vs. 35.5%, χ2=8.878, P=0.003), and the radiotherapy combined transplantation group also had a better 5-years OS rate than the non-radiotherapy combined transplantation group (45.8% vs. 37.3%, χ2=5.945, P=0.015). Univariate survival analysis showed lactate dehydrogenase ≥ 1 500 U/L, the amplification of MYCN, the metastatic sites of orbit, canalis spinalis and pleura were associated with poor prognosis of newly diagnosed high-risk NB patients (χ2=21.064, 13.601, 3.998, 6.183, 15.307, all P<0.05). The amplification of MYCN and the metastatic sites of pleura were risk factors for prognosis of newly diagnosed high-risk NB patients by Cox regression models (HR=1.896,1.100, 95%CI: 1.113-3.231, 1.020-1.187, both P<0.05).@*Conclusions@#The prognosis is unfavorable in high-risk group NB patients with BM metastasis. Radiotherapy combined with transplantation can further improve the prognosis of these patients. The amplification of MYCN and the metastatic sites of pleura were the poor prognostic factors for high-risk NB patients with bone marrow metastasis.
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Objective To analyze the changes in nutritional status changes of pediatric acute lymphoblastic leukemia (ALL) patients before and after chemotherapy and to evaluate the effects of enteral nutritional support on the states of nutrition and complication of chemotherapy.Methods Sixty-two newly diagnosed ALL patients from Novem-ber 2012 to December 2013 in Center of Hematology,Beijing Children's Hospital Affiliated to Capital Medical University were enrolled in this study.Patients were randomly assigned into an intervention group and a control group.During the induction and the early consolidation chemotherapy,the control group was given routine low fat diet routinely,and the intervention group was given Nestle peptamen [20 mL/(kg · d)] as the enteral nutritional support,meanwhile the routine low fat diet was also given.Changes in the nutritional status before and after chemotherapy and effectiveness of nutritional complementary therapy for preventing the chemotherapy complications were analyzed.Results (1) Basic information:there was no significant difference in age,gender,risk group,anthropometry,albumin and pre-albumin level between 2 groups before chemotherapy.(2)Nutritional status:the rate of malnutrition was 11.3 ％,and skinniness cases under 5 years of age occupied 10.0％.In the control group,the Z scores of W/H (weight-for-length/height,t =3.160,P =0.040),the Z scores of BMI (body mass index,t =3.490,P =0.010) and the albumin level(t =-1.805,P ＜0.001) decreased after chemotherapy,and the difference was statistically significant.On the other hand,the Z scores of W/H and BMI kept stable after chemotherapy in the intervention group,the albumin level raised from (40.53 ±3.96) g/L to (44.36 ± 3.31) g/L (t =-4.500,P ＜ 0.001) and the pre-albumin level raised from (126.55 ± 39.28) g/L to (189.55 ± 51.81) g/L(t =2.710,P =0.010),which was of statistical difference.The albumin level(t =5.020,P ＜ 0.001),pre-albumin level (t =3.036,P =0.040) and the Z scores of W/H (t =2.790,P =0.010),BMI (t =3.370,P ＜ 0.001),weight for age (W/A,t =2.830,P =0.010) were all higher in the intervention group,and the differences in statistical significance were found.(3)Side effects of chemotherapy:patients in the intervention group had higher hemoglobin (t =2.070,P =0.043) and platelet (Z =-2.19,P =0.033) level during the chemotherapy which induce less platelet [(0.50 ± 1.00) U vs (2.00 ± 2.00) U ; Z =-3.53,P =0.003] and red blood cells [(3.87 ± 2.01) U vs (5.25 ± 1.87) U ;t =-2.810,P =0.007] transfusion.Period of neutrophil deficiency [(15.67 ± 8.85)d vs (25.94 ±8.72) d;t =-4.601,P ＜0.001]was also shorter than that in the control group.Other complications had no difference between two groups exclude mild liver function abnormality was found more in the controls(x2 =6.680,P =0.010).(4)Safety:the complete remission rate 15 days after chemotherapy was 83.3％ in the intervention group and 81.2％ in the control group (x2 =0.046,P =0.830).All patients got complete remission on day 33.There was no significant difference.No pancreatitis happened in both groups during the chemotherapy.Conclusions Malnutrition rate is high among newly diagnosed ALL pediatric patients,and the nutritional status will deteriorate during the chemotherapy.Enteral nutritional support contributes to maintaining the stability of nutritional status.Enteral nutritional support improves the tolerance of hematopoietic system to chemotherapy.The effect for other complications remains to be confirmed by more extensive study in future.Nestle peptamenas enteral nutritional support productions are safe for ALL patients undergoing chemotherapy.